Saethre-Chotzen syndrome (SCS) is a type of acro-cephalo-syndactyly (ACS) syndrome. ACS syndromes are a group of autosomal dominant syn- dromes, in which craniosynostosis is associated with acrocephaly and syndactyly. Main features

Saethre-Chotzen syndrome (SCS) is a type of acro-cephalo-syndactyly (ACS) syndrome. ACS syndromes are a group of autosomal dominant syndromes, in which craniosynostosis is associated with acrocephaly and syndactyly. Main features characterizing SCS, also known as ACS III, are premature fusion of the coronal sutures leading to skull deformation, facial dysmorphism, syndactyly, skeletal deformity and congenital heart malformations. Presence of mid-facial hypoplasia is expected to predispose the patient to a difficult airway (Table 1). These patients can present with multiple surgical and medical complaints. Most of them will undergo surgery and anesthesia at least once in their life time for correction of craniofacial, orthopedic, ophthalmic, or cardiac lesions apart from incidental surgical conditions. We here describe a child with diagnosed SCS, who underwent squint surgery under general anesthesia, and also discuss the associated anesthetic concerns thereof.